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Pulmonary hypertension in children with sickle cell disease

Warren A Zuckerman1, Erika B Rosenzweig

  • 1Columbia University Medical Center, 3959 Broadway, New York, NY 10032, USA. wz2116@columbia.edu

Expert Review of Respiratory Medicine
|April 23, 2011
PubMed

Insights

Pediatric sickle cell disease patients show high rates of elevated tricuspid regurgitation jet velocity, indicating pulmonary hypertension. Further research is needed to understand its long-term impact on children with sickle cell disease.

Area of Science:

  • Cardiology
  • Hematology
  • Pediatrics

Background:

  • Pulmonary hypertension and cor pulmonale are known complications of sickle cell disease (SCD).
  • These complications were previously thought to primarily affect adults with SCD.
  • Recent pediatric studies reveal a high prevalence of elevated tricuspid regurgitation jet velocity (TRJV) in children with SCD.

Purpose of the Study:

  • To review the current literature on the prevalence, etiology, and implications of elevated TRJV in pediatric SCD patients.
  • To discuss the potential impact of elevated TRJV on mortality in children and adolescents with SCD.
  • To propose screening, diagnostic, treatment, and follow-up strategies for pulmonary hypertension in pediatric and adult SCD populations.

Main Methods:

  • Literature review of recent studies on pediatric sickle cell disease and pulmonary hypertension.
  • Analysis of findings related to elevated tricuspid regurgitation jet velocity (TRJV) in pediatric SCD cohorts.
  • Comparison of pediatric findings with existing data from adult SCD populations.

Main Results:

  • Pediatric SCD patients exhibit a prevalence of elevated TRJV comparable to adult counterparts.
  • Elevated TRJV suggests increased right-sided pressures, indicative of pulmonary hypertension.
  • The long-term mortality implications of elevated TRJV in pediatric SCD are currently unsubstantiated.

Conclusions:

  • Elevated TRJV is a significant finding in pediatric SCD, suggesting a high burden of pulmonary hypertension.
  • Further investigation is crucial to establish the mortality impact and optimize management strategies.
  • Standardized screening and management protocols are needed for both pediatric and adult SCD patients to address pulmonary hypertension.

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